Genomes and Genes
Affiliation: Yale University
- Development of the bile ducts: essentials for the clinical hepatologistMario Strazzabosco
Section of Digestive Diseases, Yale University, New Haven, CT, USA
J Hepatol 56:1159-70. 2012..Finally, we discuss the pathogenic aspects of the most important primary cholangiopathies related to altered biliary development, i.e. polycystic and fibropolycystic liver diseases, Alagille syndrome...
- Differentially expressed adenylyl cyclase isoforms mediate secretory functions in cholangiocyte subpopulationMario Strazzabosco
Section of Digestive Diseases, Department of Internal Medicine, Yale University School of Medicine and Liver Center, New Haven, CT 06520, USA
Hepatology 50:244-52. 2009..Treatment with lipopolysaccharide or alpha-naphthylisothiocyanate increased expression of AC7 and sAC but decreased expression of the other ACs...
- Functional anatomy of normal bile ductsMario Strazzabosco
Department of Internal Medicine, Yale University, New Haven 06504, Connecticut, USA
Anat Rec (Hoboken) 291:653-60. 2008..Functional interactions with other branching systems, such as nerve and vascular structures, are crucial in the modulation of the different cholangiocyte functions...
- Foxa1 and Foxa2 regulate bile duct development in miceMario Strazzabosco
Liver Center and Department of Internal Medicine, Yale University, USA
J Hepatol 52:765-7. 2010..Our data suggest that Foxa1/2 function as terminators of bile duct expansion in the adult liver through inhibition of IL-6 expression...
- Polycystic liver diseases: congenital disorders of cholangiocyte signalingMario Strazzabosco
Liver Center and Section of Digestive Diseases, Department of Internal Medicine, School of Medicine, Yale University, New Haven, Connecticut 06520, USA
Gastroenterology 140:1855-9, 1859.e1. 2011..Each pathway represents a potential target for therapies aimed at reducing disease progression...
- Cyclic AMP/PKA-dependent paradoxical activation of Raf/MEK/ERK signaling in polycystin-2 defective mice treated with sorafenibCarlo Spirli
Liver Center and Section of Digestive Diseases, Department of Internal Medicine, Yale University, New Haven, CT 06520, USA
Hepatology 56:2363-74. 2012....
- Mammalian target of rapamycin regulates vascular endothelial growth factor-dependent liver cyst growth in polycystin-2-defective miceCarlo Spirli
Section of Digestive Diseases, Department of Internal Medicine, Yale University, New Haven, CT 06520, USA
Hepatology 51:1778-88. 2010..Phosphorylation of the mTOR-dependent kinase P70S6K was significantly reduced by PKA inhibitor 14-22 amide and by the mitogen signal-regulated kinase inhibitor U1026...
- Altered store operated calcium entry increases cyclic 3',5'-adenosine monophosphate production and extracellular signal-regulated kinases 1 and 2 phosphorylation in polycystin-2-defective cholangiocytesCarlo Spirli
Department of Internal Medicine, Liver Center and Digestive Diseases Section, Yale University, New Haven, CT 06520, USA
Hepatology 55:856-68. 2012..g., the Orai1 channel) are unchanged. In Pkd2KO cells, ER-Ca(2+) depletion increases cAMP and PKA-dependent ERK1/2 activation and both are inhibited by STIM1 inhibitors or by silencing of adenylyl cyclase type 6 (AC6)...
- ERK1/2-dependent vascular endothelial growth factor signaling sustains cyst growth in polycystin-2 defective miceCarlo Spirli
Section of Digestive Diseases, Department of Internal Medicine, Yale University, New Haven, Connecticut 06520, USA
Gastroenterology 138:360-371.e7. 2010..We investigated the effects of VEGF on liver cyst growth and autocrine VEGF signaling in mice with Pkd1 and Pkd2 conditional knockouts...
- Loss of CFTR affects biliary epithelium innate immunity and causes TLR4-NF-κB-mediated inflammatory response in miceRomina Fiorotto
Section of Digestive Diseases, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut 06520, USA
Gastroenterology 141:1498-508, 1508.e1-5. 2011..We studied the effects of CFTR deficiency on Toll-like receptor 4 (TLR4)-mediated responses of the biliary epithelium to endotoxins...
- Protein kinase A-dependent pSer(675) -β-catenin, a novel signaling defect in a mouse model of congenital hepatic fibrosisCarlo Spirli
Liver Center and Section of Digestive Diseases, Department of Internal Medicine, Yale University, New Haven, CT, USA
Hepatology 58:1713-23. 2013..Cell migration (Boyden chambers) was significantly higher in cholangiocytes obtained from Pkhd1(del4/del4) and was inhibited by: (1) PKI, (2) silencing β-catenin (siRNA), and (3) the Rac-1 inhibitor NSC 23766...
- Notch signaling regulates tubular morphogenesis during repair from biliary damage in miceRomina Fiorotto
Liver Center and Section of Digestive Diseases, Department of Internal Medicine, Yale University School of Medicine, New Haven, CT 06520, USA
J Hepatol 59:124-30. 2013..We hypothesized that the morphogenetic role of Notch signaling is maintained during the repair process and have addressed this hypothesis using pharmacologic and genetic models of defective Notch signaling...
- The cystic fibrosis transmembrane conductance regulator controls biliary epithelial inflammation and permeability by regulating Src tyrosine kinase activityRomina Fiorotto
Section of Digestive Diseases, Liver Center, Yale University, New Haven, CT
Hepatology 64:2118-2134. 2016....
- Adenylyl Cyclase 5 links changes in calcium homeostasis to cAMP-dependent cyst growth in Polycystic Liver DiseaseCarlo Spirli
Section of Digestive Diseases, Yale University, New Haven, Connecticut, USA
J Hepatol . 2016....
- Posttranslational regulation of polycystin-2 protein expression as a novel mechanism of cholangiocyte reaction and repair from biliary damageCarlo Spirli
Section of Digestive Diseases, Yale University, New Haven, CT
Hepatology 62:1828-39. 2015..Treatment of 3,5-diethoxycarbonyl-1,4-dihydrocollidine mice and of Mdr2(-/-) mice with the proteasome inhibitor bortezomib restored PC2 expression and significantly reduced the ductular reaction, fibrosis, and phosphorylated ERK1/2...
- Type I autoimmune hepatitis presenting with acute liver failure in the setting of wild mushroom ingestionJason N Rogart
Department of Medicine, Section of Digestive, Yale University School of Medicine, New Haven, CT 06520 8019
J Clin Gastroenterol 42:662-6. 2008..Here, we report the unusual case of a woman in her seventh decade who presented with acute liver failure as her initial manifestation of autoimmune hepatitis, and who had been a long-time ingestor of hand-picked, wild mushrooms...
- Stimulation of nuclear receptor peroxisome proliferator-activated receptor-γ limits NF-κB-dependent inflammation in mouse cystic fibrosis biliary epitheliumRoberto Scirpo
Section of Digestive Diseases, Liver Center, Yale University, New Haven, CT
Hepatology 62:1551-62. 2015..Stimulation of PPAR-γ in vivo (rosiglitazone) significantly attenuates biliary damage and inflammation in Cftr-knockout mice exposed to a dextran sodium sulfate-induced portal endotoxemia...
- Revisiting Epithelial-to-Mesenchymal Transition in Liver Fibrosis: Clues for a Better Understanding of the "Reactive" Biliary Epithelial PhenotypeLuca Fabris
Department of Molecular Medicine, University of Padua School of Medicine, Viale G Colombo 3, 35131 Padua, Italy Liver Center, Section of Digestive Diseases, Yale University, TAC Building, 333 Cedar Street, New Haven, CT 06520, USA
Stem Cells Int 2016:2953727. 2016..Understanding microenvironmental cues and signaling perturbations promoting these changes in DRC may help to identify potential targets for new antifibrotic therapies in cholangiopathies. ..
- Ursodeoxycholic acid stimulates cholangiocyte fluid secretion in mice via CFTR-dependent ATP secretionRomina Fiorotto
Department of Internal Medicine, Section of Digestive Diseases, Yale University School of Medicine and Liver Center, New Haven, Connecticut, USA
Gastroenterology 133:1603-13. 2007..Ursodeoxycholic acid (UDCA) is widely used for cholangiopathy treatment, but its effects on cholangiocyte secretory functions remain unclear and are the subject of this study...
- Epithelial expression of angiogenic growth factors modulate arterial vasculogenesis in human liver developmentLuca Fabris
CeLiveR, Center for Liver Research, Ospedali Riuniti di Bergamo, Bergamo, Italy
Hepatology 47:719-28. 2008..In early postnatal Hnf6(-/-) mice, VEGF-expressing ductal plates failed to incorporate into the portal mesenchyma, resulting in severely altered arterial vasculogenesis...
- Defective regulation of cholangiocyte Cl-/HCO3(-) and Na+/H+ exchanger activities in primary biliary cirrhosisSaida Melero
Division of Hepatology and Gene Therapy, University of Navarra, Pamplona, Spain
Hepatology 35:1513-21. 2002....
- Cytokine-stimulated nitric oxide production inhibits adenylyl cyclase and cAMP-dependent secretion in cholangiocytesCarlo Spirli
Department of Medical and Surgical Sciences, University of Padova, Italy
Gastroenterology 124:737-53. 2003..The biliary epithelium is involved both in bile production and in the inflammatory/reparative response to liver damage. Recent data indicate that inflammatory aggression to intrahepatic bile ducts results in chronic progressive cholestasis...
- Ursodeoxycholate further increases bile-duct cell proliferative response induced by partial bile-duct ligation in ratsMichele Barone
Section of Gastroenterology, D E T O, University of Bari, Italy
Virchows Arch 444:554-60. 2004....
- Glibenclamide stimulates fluid secretion in rodent cholangiocytes through a cystic fibrosis transmembrane conductance regulator-independent mechanismCarlo Spirli
Department of Meidcal and Surgical Sciences, University of Padova, Italy
Gastroenterology 129:220-33. 2005..We studied the molecular mechanisms underlying this effect and whether glibenclamide could restore cholangiocyte secretion in cystic fibrosis...
- Thiazolidinedione treatment inhibits bile duct proliferation and fibrosis in a rat model of chronic cholestasisFabio Marra
Dipartimento di Medicina Interna, University of Florence, Viale Morgagni 85, Florence I 50134, Italy
World J Gastroenterol 11:4931-8. 2005....
- Effects of angiogenic factor overexpression by human and rodent cholangiocytes in polycystic liver diseasesLuca Fabris
Center for Liver Research, Gastroenterology Division, Ospedali Riuniti, Bergamo, Italy
Hepatology 43:1001-12. 2006..Supplementary material for this article can be found on the HEPATOLOGY website (http://interscience.wiley.com/jpages/0270-9139/suppmat/index.html)...
- Treatment with pegylated interferon and ribavirin for hepatitis C virus-associated severe cryoglobulinemia in a liver/kidney transplant recipientMarzia Montalbano
Section of Gastroenterology and CeLiverR, Ospedali Riuniti di Bergamo, Bergamo, Italy
J Clin Gastroenterol 41:216-20. 2007..Treatment with pegylated-IFN-alpha2b and RBV for 12 months cured the cryoglobulinemic vasculitis and allowed the sustained eradication of HCV with no significant changes in kidney function...
- Analysis of liver repair mechanisms in Alagille syndrome and biliary atresia reveals a role for notch signalingLuca Fabris
CeLiveR, Gastroenterology and Liver Transplant Unit, Ospedali Riuniti di Bergamo, Bergamo, Italy
Am J Pathol 171:641-53. 2007....
- Morphological and functional features of hepatic cyst epithelium in autosomal dominant polycystic kidney diseaseDomenico Alvaro
Department of Clinical Medicine, University of Rome Sapienza, via R Rossellini 51, 00137 Rome, Italy
Am J Pathol 172:321-32. 2008..Our findings could explain the role of estrogens in accelerating the progression of ADPKD and may suggest a potential benefit of therapeutic strategies based on estrogen antagonism...
- Timing of reinfection and mechanisms of hepatocellular damage in transplanted hepatitis C virus-reinfected liverGiorgio Ballardini
Department of Internal Medicine, Azienda Ospedaliera and University of Bologna, Bologna, Italy
Liver Transpl 8:10-20. 2002....